From the Guidelines
The diagnostic criteria for Neuro-Behçet's disease (NBD) require a combination of clinical, radiological, and laboratory findings, including recurrent oral ulcers plus at least two of the following: genital ulcers, eye lesions, skin lesions, or a positive pathergy test, as well as neurological symptoms and characteristic MRI findings. To diagnose NBD, a patient must first meet the criteria for Behçet's disease, which was established by the International Group for Behçet’s disease 1. For the neurological component, the criteria include:
- Neurological symptoms consistent with NBD, such as headache, cognitive dysfunction, pyramidal signs, brainstem symptoms, or psychiatric manifestations.
- Characteristic MRI findings, typically showing T2-weighted hyperintense lesions in the brainstem, basal ganglia, or white matter, with large diencephalic and infratentorial lesions being more specific for NBD 1.
- Cerebrospinal fluid (CSF) analysis showing pleocytosis (>5 cells/mm³) with neutrophil predominance in acute cases, or lymphocyte predominance in chronic cases.
- Exclusion of other neurological conditions that could explain the symptoms.
- Response to immunosuppressive therapy, which can be supportive of the diagnosis. It's essential to note that not all criteria need to be met for a diagnosis, and clinical judgment plays a crucial role, as highlighted in the guidelines for the diagnosis and management of patients with thoracic aortic disease 1. The presence of systemic Behçet's disease features, typical MRI findings, and appropriate CSF changes in the context of neurological symptoms are often sufficient for diagnosis, and early diagnosis and treatment are essential to prevent long-term neurological damage in NBD.
From the Research
Diagnostic Criteria for Neuro-Behcet's Disease
The diagnostic criteria for Neuro-Behcet's disease, a form of Behcet's disease, involve a combination of clinical manifestations and the presence of specific symptoms. According to 2, the international study group for Behcet's disease has defined a set of diagnostic criteria that require the presence of recurrent oral ulcers plus two of the following:
- Recurrent genital ulcerations
- Typical defined eye lesions
- Typical defined skin lesions
- A positive pathergy test
Clinical Features and Diagnosis
Behcet's disease is characterized by recurrent mucocutaneous lesions and major organ disease, including ocular, neurologic, vascular, and gastrointestinal manifestations 3. The diagnosis of Behcet's disease is mainly based on clinical manifestations after ruling out other potential causes. There are no specific laboratory, histopathologic, or genetic findings for the diagnosis of Behcet's disease.
Neuro-Behcet's Disease Diagnosis
Neuro-Behcet's disease is defined as a combination of neurologic symptoms and/or signs in a patient with Behcet's disease 4. The diagnosis of Neuro-Behcet's disease involves the presence of neurologic symptoms, such as brainstem syndrome, multiple-sclerosis like presentations, movement disorders, meningoencephalitic syndrome, myelopathic syndrome, cerebral venous sinus thrombosis, and intracranial hypertension. Central nervous involvement falls into parenchymal and non-parenchymal subtypes.
Diagnostic Tools and Tests
The pathergy test is the only diagnostic test used as diagnostic or classification criteria for Behcet's disease 3. Other diagnostic tools, such as brain magnetic resonance imaging (MRI), MR venography (MRV), and computerized tomographic venography (CTV), can be used to diagnose cerebral venous sinus thrombosis and other neurologic manifestations of Behcet's disease 4.
Expert Consensus Recommendations
Expert consensus recommendations have been developed for the diagnosis and management of Neuro-Behcet's disease, including consensus criteria for diagnosis and recommendations on when to consider Neuro-Behcet's disease in a neurological patient 5. These recommendations also include treatment guidelines for parenchymal Neuro-Behcet's disease and cerebral venous thrombosis, as well as the use of disease-modifying therapies.